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Pustular psoriasis: Management

Pustular psoriasis: Management
Author:
Robert E Kalb, MD
Section Editor:
Kristina Callis Duffin, MD
Deputy Editor:
Abena O Ofori, MD
Literature review current through: May 2025. | This topic last updated: Jun 27, 2025.

INTRODUCTION — 

Pustular psoriasis is an uncommon subtype of psoriasis that may present as a generalized pustular skin eruption (generalized pustular psoriasis [GPP] (picture 1A-D)) or a localized pustular skin eruption (eg, acrodermatitis continua of Hallopeau (picture 2A-C)).

Treatment of GPP aims to improve skin manifestations, alleviate associated systemic symptoms, and minimize risk for life-threatening systemic complications. Treatment of localized pustular psoriasis focuses on minimizing skin manifestations and bothersome or disabling symptoms.

The management of GPP and acrodermatitis continua of Hallopeau will be reviewed here. The management of GPP in pregnant individuals is reviewed separately. Palmoplantar pustulosis is also discussed separately.

●(See "Dermatoses of pregnancy", section on 'Pustular psoriasis of pregnancy'.)

●(See "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis".)

●(See "Palmoplantar pustulosis: Treatment".)

The management of forms of psoriasis is also reviewed separately.

●(See "Erythrodermic psoriasis in adults".)

●(See "Chronic plaque psoriasis in adults: Overview of management".)

●(See "Chronic plaque psoriasis in adults: Treatment of disease amenable to topical therapy".)

●(See "Chronic plaque psoriasis in adults: Treatment of disease requiring phototherapy or systemic therapy".)

●(See "Psoriasis in children: Management of chronic plaque psoriasis".)

●(See "Guttate psoriasis".)

●(See "Nail psoriasis".)

DISEASE OVERVIEW

Generalized pustular psoriasis — Generalized pustular psoriasis (GPP) is characterized by the development of a widespread eruption of pustules and erythematous plaques (picture 1B and picture 1A, 1C-D). A preceding history of psoriasis may or may not be present.

●Acute GPP – The acute variant (also known as generalized pustular psoriasis of von Zumbusch) is characterized by the sudden onset of a pustular eruption accompanied by fever and malaise. Laboratory abnormalities, such as leukocytosis, an elevated erythrocyte sedimentation rate, hypocalcemia and other electrolyte abnormalities, hypoalbuminemia, and elevated liver enzymes, are common. In addition, serious complications, including sepsis and hepatic, respiratory, or renal dysfunction, can occur [1-4]. (See "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis", section on 'Generalized pustular psoriasis'.)

●Generalized annular pustular psoriasis – GPP may also present as a less acute disorder in which patients develop widespread, annular or figurate, erythematous plaques with peripheral pustules and scale (generalized annular pustular psoriasis) [4,5]. Pain and fever may accompany these cutaneous manifestations. Generalized annular pustular psoriasis is a common presentation of pustular psoriasis in children. (See "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis", section on 'Clinical manifestations' and "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis", section on 'Epidemiology'.)

There is no cure for GPP, and recurrences are common [4]. Thus, long-term treatment is often required to minimize recurrences of disease.

Acrodermatitis continua of Hallopeau — Acrodermatitis continua of Hallopeau is a chronic, localized form of pustular psoriasis. The condition typically presents with erythema and sterile pustules on the fingers or toes (picture 2A and picture 2B-C). (See 'Acrodermatitis continua of Hallopeau' below and "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis", section on 'Acrodermatitis continua of Hallopeau'.)

Palmoplantar pustulosis — Palmoplantar pustulosis is a chronic condition characterized by the development of yellow to brown pustules, scale, and patchy erythema on the palms or soles. It is controversial whether palmoplantar pustulosis is a localized variant of pustular psoriasis (palmoplantar pustular psoriasis) or a separate entity. (See "Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis".)

The management of palmoplantar pustulosis is reviewed separately. (See "Palmoplantar pustulosis: Treatment".)

ACUTE GENERALIZED PUSTULAR PSORIASIS

Approach to therapy — Limited data on treatments for acute generalized pustular psoriasis (GPP) complicate determining the optimal approach to treatment (picture 1B and picture 1A, 1C-D) [6].

The approach described in this topic reflects our preferred approach based on a review of the literature and clinical experience; other approaches may also be reasonable (algorithm 1). In Japan, multiple therapies have regulatory approval for the treatment of GPP.

Important interventions — Our approach to the treatment of acute GPP consists of the following key steps:

●Determine need for hospitalization and supportive care – Patients with acute GPP usually appear systemically ill, and admission to the hospital often is necessary to ensure adequate supportive care. The decision to hospitalize a patient is made based on global consideration of the severity of illness, vital sign stability, fluid and electrolyte status, and concern for systemic infection.

Supportive skin care measures may help to soothe skin symptoms in patients with GPP. Moisturizers, wet wraps, and/or oatmeal baths can be beneficial [1].

●Identify and discontinue the causative drug (in drug-induced cases) – The withdrawal or administration of a variety of drugs has been linked to the development of GPP. In general, the causative agent should be discontinued, provided this can be done safely. There is at least one report of successful management of GPP without discontinuation of the inciting medication [7].

Systemic glucocorticoids are commonly cited contributors [4]. However, there is insufficient evidence to confirm the best way to discontinue systemic glucocorticoids in patients with glucocorticoid-induced GPP. Options include maintaining the glucocorticoid dose until disease control is achieved with other therapies and continuing to taper the systemic glucocorticoid during the initiation of GPP therapy. (See "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis", section on 'Precipitating factors'.)

Patients in whom GPP was precipitated by an antipsoriatic drug may be best managed with an agent with a different mechanism of action. For example, the tumor necrosis factor (TNF)-alpha inhibitor infliximab would be a less favorable choice for a patient with adalimumab-induced GPP.

●Initiate treatment to control skin disease – Systemic therapy is the primary mode of treatment for acute GPP. Topical therapies are impractical for widespread disease and primarily serve as adjunctive treatments. (See 'Adjunctive topical therapy' below.)

●Manage extracutaneous complications – Extracutaneous complications, such as sepsis or internal organ dysfunction, should be managed appropriately. (See "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis", section on 'Clinical course'.)

Treatment selection — Data on most treatments for acute GPP primarily come from retrospective studies, case reports, and expert opinion, with most studies originating from a single country (Japan). Few randomized trials have been performed. Interpretation of the available data is further compromised by the use of various grading systems for disease severity and treatment response [8].

●Our approach – Spesolimab is our preferred treatment for acute GPP in nonpregnant adults (algorithm 1). Our choice of spesolimab is based on randomized trial data that support the efficacy of spesolimab therapy for acute flares and flare prevention. It is unclear whether spesolimab is more effective than other therapies. (See 'Spesolimab' below.)

When spesolimab therapy for an acute GPP flare is not feasible or effective, we select another fast-acting antipsoriatic drug, such as a biologic IL-17 or IL-23 inhibitor, infliximab, or cyclosporine. We typically select a biologic anti-IL-17 or anti-IL-23 inhibitor because of the favorable adverse effect profiles of these drugs. (See 'IL-17 inhibitors' below and 'IL-23 inhibitors' below.)

However, because rapid initiation of therapy is important for patients with severe, acute GPP, treatment availability often influences treatment. We sometimes find it necessary to use cyclosporine or infliximab when we cannot obtain our preferred therapies quickly. (See 'Alternatives for acute flares' below.)

Children — Efficacy and safety data on treatments for acute GPP in children are limited.

Treatment selection is primarily based on clinical experience and drug safety data from studies in chronic plaque psoriasis [9,10].

●Our approach – Similar to adults, rapid-acting antipsoriatic therapies are preferred for acute GPP flares in children. We tend to prefer the IL-17 inhibitors secukinumab or ixekizumab for children at least six years of age, because of demonstrated safety and efficacy in chronic plaque psoriasis trials that have included children. (See 'IL-17 inhibitors' below.)

In addition, secukinumab appeared to improve GPP more quickly than ustekinumab in a retrospective study of 33 children and 32 adults given one of these drugs for GPP [11] (see 'IL-23 inhibitors' below). In another retrospective study of 20 children treated with secukinumab and 16 children treated with acitretin, the average time to clinical improvement of GPP was shorter in children given secukinumab [12]. Improvement of pediatric GPP with secukinumab has also been documented in case reports and case series [13-16].

However, other approaches may be reasonable.

Pediatric dosing regimens for secukinumab and ixekizumab for GPP resemble the dosing regimens used for chronic plaque psoriasis.

•Secukinumab for children ≥6 years of age – For children <50 kg, 75 mg given at weeks 0, 1, 2, 3, and 4 and then every 4 weeks; for children ≥50 kg, 150 mg given at weeks 0, 1, 2, 3, and 4 and then every 4 weeks.

•Ixekizumab for children ≥6 years of age – For children <25 kg, 40 mg at week 0 and then 20 mg every 4 weeks; for children 25 to 50 kg, 80 mg at week 0 and then 40 mg every 4 weeks; for children >50 kg, 160 mg at week 0 and then 80 mg every 4 weeks.

●Alternatives – Limited data suggest that spesolimab may be a reasonable alternative for some patients. A single intravenous dose of spesolimab (450 mg for children 20 to 50 kg and 900 mg for children ≥50 kg) appeared effective and well tolerated in children with GPP (aged 4 to 12 years) in a case series [17]. Additionally, the Effisayil 2 trial, which assessed the efficacy of subcutaneous spesolimab for the prevention of GPP flares, included six children (ages 14 to 17 years) [18]. (See 'Spesolimab' below.)

•Spesolimab – Dosing for children at least 12 years of age and weighing at least 40 kg is identical to adult dosing. (See 'Spesolimab' below.)

As with adults, additional treatment options for acute flares in children include cyclosporine [19-25] and infliximab [26-28].

•Cyclosporine – At 1 to 3 mg/kg per day

•Infliximab – At 5 mg/kg at weeks 0, 2, and 6, then every 6 to 8 weeks if continuation of therapy is needed

Examples of other therapies with reports of benefit in children include oral retinoids [29-35], methotrexate [33,36,37], etanercept [26,27,38-40], adalimumab [41-44], ustekinumab [11], infliximab with methotrexate [38] narrowband ultraviolet B (UVB) phototherapy with systemic therapy [20,45], and anakinra [46]. Pregnancy is contraindicated during acitretin treatment and for three years after drug discontinuation, warranting caution with the use of acitretin in children and adolescents of or approaching childbearing potential.

Patients with IL36RN mutations — Some cases of GPP (or acute generalized exanthematous pustulosis [AGEP]) represent a genetic autoinflammatory disease based on mutations in the IL36RN gene, which encodes the IL-36 receptor antagonist. This disease is known as DITRA. (See "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis" and "Autoinflammatory diseases mediated by inflammasomes and related IL-1 family cytokines (inflammasomopathies)", section on 'Deficiency of the IL-36 receptor antagonist (DITRA)' and "Acute generalized exanthematous pustulosis (AGEP)".)

Many systemic therapies for acute GPP, such as spesolimab, IL-17 inhibitors, IL-23 inhibitors, TNF inhibitors, and acitretin appear to be beneficial both in the presence and absence of the IL36RN mutation [18,40,47-51].

Pregnant patients — The treatment of GPP in pregnant individuals is reviewed separately. (See "Dermatoses of pregnancy", section on 'Pustular psoriasis of pregnancy'.)

Response assessment — The physical examination is the primary method of assessing the response to therapy. Initial signs of a good response include the cessation of new pustule formation, the healing of pustules, and a reduction in erythema.

Other disease manifestations, such as systemic symptoms and laboratory abnormalities, are also expected to improve with treatment.

In the event of a poor response, the clinician should assess for the accuracy of the GPP diagnosis, the potential for ongoing disease triggers (eg, medications, infections), and the presence of secondary or concomitant causes of skin pustules (eg, bacterial or fungal infections) prior to assuming treatment-resistant GPP. (See "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis", section on 'Diagnosis' and "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis", section on 'Differential diagnosis'.)

Treatment duration — The course of GPP is unpredictable. We typically continue treatment after control of an acute flare because of the possibility of flare recurrence and the potentially life-threatening nature of GPP. However, in select cases, such as an initial episode of GPP with a clear inciting trigger that can be avoided, we consider stopping treatment to assess whether the disease will recur a reasonable alternative to continued treatment.

For patients who will continue treatment, we often continue the same therapy that successfully treated an acute flare, provided the treatment was tolerated (algorithm 1). We typically transition patients on cyclosporine to another drug because of a concern for the risk of serious adverse effects with long-term cyclosporine therapy. (See "Pharmacology of calcineurin inhibitors", section on 'Side effects'.)

The clinical scenario and shared decision-making with the patient guide the approach to treatment duration. In general, we consider trials of reducing therapy reasonable for patients who achieve long-term, stable remissions (eg, 3 to 6 months). We tend to taper nonbiologic therapies but stop biologic therapies with the plan to restart biologic therapy if needed.

A more cautious approach to treatment cessation is prudent for patients with a history of severe disease. In these patients, extended treatment courses may be reasonable.

Preferred treatments (adults) — Our preferred treatments for acute GPP in nonpregnant adults include spesolimab and biologic IL-17 or IL-23 inhibitors (algorithm 1). We aim to treat with spesolimab when feasible.

Spesolimab — Spesolimab is a monoclonal antibody that targets the IL-36 receptor.

In 2022, the US Food and Drug Administration (FDA) approved intravenous spesolimab for the treatment of GPP flares in adults. In 2024, the FDA updated the approval of spesolimab for GPP to include subcutaneous administration for patients not experiencing a flare and spesolimab therapy for children at least 12 years of age and weighing at least 40 kg [52].

Of note, long-term safety data for spesolimab are limited.

●Administration and precautions – Acute GPP flares are typically treated with intravenous spesolimab. Subcutaneous spesolimab is generally used for long-term disease suppression in patients with non-flaring or stable GPP.

Standard spesolimab regimens for patients at least 12 years of age and weighing at least 40 kg are:

•Intravenous spesolimab for acute flares – A 900 mg single dose infused over 90 minutes.

Improvement can occur as early as the first week of treatment. If flare symptoms persist, a second 900 mg dose can be given after one week. If the response is inadequate after two doses of spesolimab, and other reasons for treatment failure are not identified (eg, incorrect diagnosis), we proceed to other treatments. (See 'Response assessment' above.)

•Subcutaneous spesolimab for flare prevention – A 300 mg subcutaneous dose given every four weeks.

For patients transitioning from intravenous spesolimab, the first subcutaneous dose is given four weeks after the last intravenous dose. For patients not transitioning from intravenous spesolimab, a 600 mg subcutaneous loading dose is given four weeks prior to starting the 300 mg dose regimen.

Spesolimab may increase the risk of infection. Screening for tuberculosis is advised prior to treatment. Other adverse events have been reported, such as injection site reactions with subcutaneous spesolimab and drug reaction with eosinophilia and systemic symptoms (DRESS) [53]. (See "Spesolimab: Drug information".)

●Efficacy – Randomized trials support efficacy of spesolimab for GPP.

•Flares – In a 12-week, phase 2 trial, 53 adults with a moderate to severe GPP flare were randomly assigned in a 2:1 ratio to receive a single 900 mg subcutaneous dose of spesolimab or placebo on day 1, with options for open-label spesolimab on day 8 based on response and subsequent open-label spesolimab as a rescue treatment [53]. At day 8, 19 of 35 patients (54 percent) in the spesolimab group achieved a GPP Physician Global Assessment (GPPGA) pustulation subscore of 0 (no visible pustules) compared with only 1 of 18 (6 percent) in the placebo group (difference 32 percentage points, 95% CI 2-53). At week 12, 21 of 35 patients randomized to spesolimab (60 percent) had a GPPGA pustulation subscore of 0 [54].

Infections occurred in 17 percent of patients in the spesolimab group and 6 percent in the placebo group in week 1. Over 12 weeks, serious adverse events occurred in 6 of 51 patients (12 percent) who received at least one dose of spesolimab, including two patients with drug reaction with eosinophilia and systemic symptoms (DRESS). (See "Drug reaction with eosinophilia and systemic symptoms (DRESS)".)

•Flare prevention – In a phase 2b multicenter trial, 123 patients with GPP who had both a history of at least two prior GPP flares and a GPPGA score of 0 or 1 (clear or almost clear skin) were randomly assigned to placebo or one of three dose regimens for spesolimab [18]. Spesolimab was given subcutaneously as a low-dose (300 mg followed by 150 mg every 12 weeks), medium-dose (600 mg followed by 300 mg every 12 weeks), or high-dose (600 mg followed by 300 mg every 4 weeks) regimen. GPP flares were treated with intravenous spesolimab.

At week 48, GPP flares had occurred in 23, 29, 10, and 52 percent of patients in the low-dose, medium-dose, high-dose, and placebo groups, respectively. Compared with placebo, the high-dose regimen was more effective for reducing the time to first GPP flare (HR 0.16, 95% CI 0.05-0.54). Patients who received spesolimab and patients who received placebo had similar rates of adverse events. The most frequently documented adverse events were pustular psoriasis, psoriasis, and injection-site erythema.

IL-17 inhibitors — Clinical studies suggest benefit of the biologic IL-17 inhibitors secukinumab (an IL-17A inhibitor), ixekizumab (an IL-17A inhibitor), and brodalumab (an IL-17 receptor A inhibitor) for GPP.

Efficacy data on all IL-17 inhibitors for GPP are limited and the relative efficacy of individual IL-17 inhibitors for GPP is unclear. Based on data that support ixekizumab and bimekizumab as some of the most effective therapies for chronic plaque psoriasis, these two drugs are often our first-choice therapies (algorithm 1). However, published efficacy data for bimekizumab are limited to case reports, and in clinical practice, the selection of a specific IL-17 inhibitor for acute GPP is often dependent on which drug is available most quickly. (See "Chronic plaque psoriasis in adults: Treatment of disease requiring phototherapy or systemic therapy", section on 'Relative efficacy'.)

Optimal treatment regimens for GPP are unknown. Dosing generally reflects the standard doses for chronic plaque psoriasis. Initial improvement may be seen as early as the first one to two weeks of starting treatment [55-57].

●Precautions – Examples of potential adverse effects of IL-17 inhibitors include injection site reactions and increased risk for upper respiratory tract infection, oral candidiasis, conjunctivitis, tinea infection, and inflammatory bowel disease. Although there has been concern for an increased risk of suicidal ideation and behavior in association with brodalumab therapy, a causative relationship has not been confirmed. We avoid IL-17 inhibitor therapy in patients with inflammatory bowel disease. (See "Chronic plaque psoriasis in adults: Treatment of disease requiring phototherapy or systemic therapy", section on 'Precautions'.)

●Ixekizumab

•Administration – Typical dosing for adults is 160 mg given subcutaneously at week 0, followed by 80 mg at weeks 2, 4, 6, 8, 10, and 12. Subsequently, 80 mg is given every four weeks.

Dosing of 80 mg every two weeks has also been utilized [58]. In our experience, every-two-week dosing can be helpful when patients experience worsening disease after proceeding to an every-four-week dose interval.

•Efficacy – Ixekizumab may be beneficial based upon a 52-week, open-label study in which 78 patients with plaque psoriasis, erythrodermic psoriasis, or GPP received ixekizumab (160 mg at week 0, then 80 mg every two weeks until week 12, then 80 mg every four weeks). At week 12, four of the five patients with GPP achieved 75 percent improvement in the Psoriasis Area and Severity Index score (PASI 75), and three patients achieved 90 percent improvement (PASI 90) [56]. This level of response was sustained with continued treatment over three years [59-61]. Concomitant systemic glucocorticoid therapy equivalent to less than or equal to 10 mg per day of prednisone was permitted.

Improvement in GPP with ixekizumab is also described in a case report and case series [62,63]. In a case series of 10 patients with refractory GPP treated with ixekizumab, all nine patients available for evaluation at week 12 achieved PASI 75 [62].

●Bimekizumab

•Administration – Standard adult dosing is 320 mg given subcutaneously every 4 weeks for the first 16 weeks, followed by 320 mg given every 8 weeks.

•Efficacy – Responses to bimekizumab are documented in case reports [64,65]. In one report documenting bimekizumab therapy in two patients with GPP refractory to other therapies, marked improvement occurred within four weeks [64].

●Secukinumab

•Administration – Typical dosing for adults is 300 mg given subcutaneously once weekly at weeks 0, 1, 2, 3, and 4, followed by 300 mg every four weeks. Doses of 150 mg may be sufficient for some patients.

•Efficacy – Benefit of secukinumab is suggested in a 52-week, open-label study in which 12 adults with GPP received secukinumab (150 mg once weekly at baseline; weeks 1, 2, 3, and 4; and then every four weeks, with the option to increase the dose to 300 mg in patients who showed minimal or no improvement) [55]. Eight patients also received nonbiologic systemic therapies for psoriasis, but doses of these agents could not be increased. At week 16 (the primary endpoint), 10 patients (83 percent) received clinical global impression ratings of "very much improved" or "much improved." One patient showed no improvement, and a second patient discontinued treatment prior to 16 weeks due to a protocol deviation. Improvement was rapid, with maximum improvement achieved within three weeks. Response rates remained high at 52 weeks.

In a separate series of 13 adults and children treated with secukinumab and followed for at least six months, all had resolution of fever and nonacral pustules within 72 hours, and 92 percent of participants had clear or almost clear skin at week 24 [16].

In a retrospective study, secukinumab appeared to induce faster improvement than ustekinumab [11]. (See 'IL-23 inhibitors' below.)

●Brodalumab

•Administration – Typical brodalumab dosing for adults is 210 mg given subcutaneously at weeks 0, 1, and 2 and then every two weeks. In the United States, brodalumab must be prescribed through a Risk Evaluation and Mitigation Strategy program due to concerns regarding risk for suicidal ideation and completed suicides in treated patients. (See "Chronic plaque psoriasis in adults: Treatment of disease requiring phototherapy or systemic therapy", section on 'Brodalumab'.)

•Efficacy – A 52-week, open-label study evaluating the efficacy of the anti-IL-17 receptor A monoclonal antibody brodalumab (140 mg at day 1, weeks 1 and 2, then every two weeks, with the option to increase the dose to 210 mg) for GPP and erythrodermic psoriasis found that 11 of 12 patients with GPP achieved a clinical global impression of "improved" or "remission" by week 52 [57]. Improvement was rapid, with 9 of 12 patients achieving this status at week 2. Concomitant treatment with stable or decreasing doses of systemic methotrexate, vitamin A preparations, or glucocorticoids was permitted.

IL-23 inhibitors — Guselkumab (an IL-23 inhibitor), risankizumab (an IL-23 inhibitor), and ustekinumab (an IL-12 and IL-23 inhibitor) have been used for GPP. Whether tildrakizumab, another IL-23 inhibitor, should play a role in the treatment of GPP is unclear.

As with IL-17 inhibitors, the relative efficacy of IL-23 inhibitors for GPP is unclear. We typically select risankizumab or guselkumab based on data that support higher efficacy of these therapies for chronic plaque psoriasis compared with some other IL-23 inhibitors (algorithm 1). (See 'IL-17 inhibitors' above and "Chronic plaque psoriasis in adults: Treatment of disease requiring phototherapy or systemic therapy", section on 'Relative efficacy'.)

Dosing generally reflects the recommended doses for plaque psoriasis. Optimal treatment regimens for GPP are unknown.

Improvement with guselkumab or risankizumab can occur as early as the first week of treatment [66-68]. In our experience, responses to subcutaneous ustekinumab tend to be slower.

●Precautions – Examples of potential adverse effects of IL-23 inhibitors include infections, injection site reactions, and hypersensitivity reactions. (See "Chronic plaque psoriasis in adults: Treatment of disease requiring phototherapy or systemic therapy", section on 'IL-23 inhibitors' and "Chronic plaque psoriasis in adults: Treatment of disease requiring phototherapy or systemic therapy", section on 'IL-12/23 inhibitor'.)

●Guselkumab

•Administration – Typical dosing for adults is 100 mg given subcutaneously at weeks 0, 4, and then every 8 weeks.

•Efficacy – Guselkumab (a monoclonal antibody directed against the p19 subunit of IL-23) appeared beneficial in GPP and erythrodermic psoriasis in a 52-week, open-label study [66]. Ten patients with GPP were treated with 50 mg at weeks 0, 4, and every 8 weeks thereafter. The dose could be escalated to 100 mg at week 20 based on prespecified criteria, and patients were allowed to continue topical therapies and methotrexate. Seven of the patients with GPP achieved the primary study endpoint of treatment success (at least minimal improvement based upon a Clinical Global Impression rating at week 16), including four who achieved greater than minimal improvement. Two patients discontinued the study due to lack of response and a diagnosis of cutaneous squamous cell carcinoma. All eight patients who completed the study had treatment success at 52 weeks.

●Risankizumab

•Administration – Typical adult dosing for risankizumab is 150 mg at weeks 0, 4, and then every 12 weeks. Lower doses have also been utilized.

•Efficacy – A 180-week open-label prospective study (IMMspire study) supports efficacy of risankizumab for GPP [69]. In the study, 17 patients (8 with GPP and 9 with erythrodermic psoriasis) received open-label risankizumab (75 or 150 mg at weeks 0 and 4 and then every 12 weeks thereafter). At week 16, all patients with GPP achieved clinical response (defined as at least slightly improved) and 88 percent of these patients had achieved PASI 90. Responses were generally sustained over the course of the study for patients who continued treatment.

Nasopharyngitis was the most common adverse event (10 patients). Of four serious adverse events (gastric cancer, alcoholic liver disorder, UTI with hypoadrenalism, and urinary calculus) and one death due to ischemic heart failure, none were considered related to risankizumab treatment.

●Ustekinumab

•Administration – Dosing of ustekinumab is weight based. Typical dosing for adults ≤100 kg is 45 mg given subcutaneously at weeks 0, 4, and every 12 weeks thereafter. A 90 mg dose given in the same regimen is recommended for adults who weigh more than 100 kg.

•Efficacy – Ustekinumab appeared beneficial for GPP, but possibly with a slower onset of action than secukinumab, in a retrospective study of adults and children with moderate to severe GPP treated with either secukinumab (n = 34) or ustekinumab (n = 31) [11]. At week two, 75 percent improvement in the GPP Area and Severity Index score (GPPASI 75) occurred in more patients in the secukinumab group than in the ustekinumab group (74 versus 52 percent). Similarly, week two GPPASI 90 rates were higher in the secukinumab group (35 versus 6 percent). GPPASI 75 and 90 response rates after week four and adverse effect rates were similar between the groups.

Case reports and case series have documented efficacy of ustekinumab in acute pustular psoriasis [47,70-72]. The onset or worsening of pustular psoriasis following ustekinumab treatment has also been reported [73-76].

Alternatives for acute flares — Cyclosporine and infliximab are alternatives for acute flares of GPP (algorithm 1).

Cyclosporine — Oral cyclosporine has a long history of use for psoriasis and can induce rapid improvement of GPP.

●Administration and precautions – Effective doses of cyclosporine for adults with GPP have ranged from 2.5 to 5 mg/kg per day [77]. We typically treat severe, acute GPP with 4 to 5 mg/kg (ideal body weight) per day.

Marked improvement often occurs within the first few days of treatment [1,19,78,79]. Because side effects of cyclosporine are a concern, once disease control is achieved, we attempt to taper the dose over the course of two to three months and transition to other treatments.

Potential adverse effects of cyclosporine include hypertension, renal toxicity, and increased risk for infections and malignancy. Laboratory tests as well as blood pressure should be monitored closely during therapy. Adverse effects of cyclosporine are discussed in greater detail separately. (See "Pharmacology of calcineurin inhibitors", section on 'Side effects'.)

●Efficacy – Despite the common use of cyclosporine for severe, acute GPP, data on the efficacy of the drug are limited. A beneficial effect of cyclosporine is supported by retrospective data obtained from hospitals in Japan that suggested treatment efficacy in 60 to 70 percent of patients treated with cyclosporine alone or in conjunction with other therapies [80,81].

Infliximab — Infliximab is a TNF-alpha inhibitor that may lead to rapid improvement in GPP.

●Administration and precautions – Standard dosing of infliximab for psoriasis in adults is intravenous infusion of 5 mg/kg at weeks 0, 2, and 6, and every six to eight weeks thereafter. Marked improvement is usually evident within several days.

Infliximab may be continued for long-term management of GPP after control of acute disease is achieved. Alternatively, patients can be transitioned to other therapies.

Subcutaneous infliximab is also available but has a less established role in the treatment of GPP. We typically administer infliximab intravenously.

Potential adverse effects of infliximab include infusion reactions and increased risk for infection (including herpes zoster), malignancy, heart failure, and demyelinating disease [82,83]. Patients should be evaluated for latent tuberculosis and hepatitis B prior to initiating therapy. (See "Chronic plaque psoriasis in adults: Treatment of disease requiring phototherapy or systemic therapy", section on 'TNF-alpha inhibitors'.)

There are also reports of infliximab inducing psoriatic eruptions, including GPP [7,84,85]. The adverse effects of infliximab are reviewed separately. (See "Tumor necrosis factor-alpha inhibitors: An overview of adverse effects".)

●Efficacy – The use of infliximab for GPP is based on case reports and observational studies that suggest efficacy of this treatment [48,83,86-96].

Among 10 patients in whom flares of acute GPP were treated with infliximab in a French multicenter retrospective study, clinical remission was achieved by 8 patients (80 percent). The fast onset of infliximab was evident in the time required to achieve clearance of pustules; pustules cleared in a median of two days (range, one to eight days) [86].

A postmarketing surveillance study assessed the efficacy and safety of infliximab over six months. Among 56 patients with pustular psoriasis disease severity assessments, the proportion with moderate or severe pustular psoriasis fell from 25 and 7 percent, respectively, at baseline to 2 and 4 percent, respectively, at the final assessment, suggesting benefit [83].

The possibility that a single dose of infliximab may be sufficient is suggested by a case report documenting dramatic improvement in acute GPP within 24 hours following a single dose of infliximab followed by the initiation of methotrexate [87]. Infliximab has also been successfully used in combination with acitretin for the induction of rapid improvement while awaiting the onset of action of acitretin [97].

Additional alternatives — Acitretin (an oral retinoid) and methotrexate are additional alternatives for adults with relatively stable GPP (algorithm 1). However, methotrexate has a relatively slow onset of action and both drugs have broader side effect profiles than some biologic therapies [12]. The availability of effective, well-tolerated biologic therapies has reduced the use of acitretin and methotrexate for GPP.

Acitretin — Oral retinoids that have been used for GPP include etretinate, acitretin (a metabolite of etretinate), and isotretinoin. Acitretin is our preferred oral retinoid because clinical experience with isotretinoin for this indication is more limited, and etretinate was withdrawn from the United States market in 1998.

●Administration and precautions – Our usual starting dose for acitretin in adults is 0.75 to 1 mg/kg per day. Japanese guidelines have suggested an initial dose of etretinate of 0.5 to 1 mg/kg per day [98].

Improvement (ie, cessation of new pustule formation and initial improvement in other clinical signs) is usually noted within 7 to 10 days of oral retinoid therapy, and a complete response often requires two to three months of treatment. After disease control is achieved, the dose of acitretin can be tapered slowly to the lowest dose necessary to maintain improvement.

Examples of adverse effects of oral retinoids include xerosis, cheilitis, dry mucous membranes, hypertriglyceridemia, hair loss, liver function test abnormalities, bone changes, and visual changes.

Oral retinoids are teratogenic, and pregnancy should be avoided for three years following acitretin therapy. Therefore, the drug has a relative contraindication for people of childbearing potential.

●Efficacy – Evidence to support oral retinoid use is limited. Some support comes from a Japanese study that analyzed treatment data from 385 patients with acute GPP obtained through questionnaire responses from multiple community center hospitals [80]. The study found that among 188 patients treated with retinoids, treatment was reported as effective in 84 percent. Retinoid treatment regimens were not standardized; typically, treatment was with etretinate (1 mg/kg per day and tapered as tolerated). Many patients received retinoids in combination with other therapies.

Data from retrospective studies also suggest benefit of etretinate and acitretin [6,12,50,99,100]. There are also reports of use of acitretin and secukinumab as combination therapy [101,102].

In a series of 11 adults with GPP, isotretinoin therapy seemed beneficial for improving pustule formation in 10 patients, although monotherapy with the drug did not seem to be effective for clearing all lesions [103]. Isotretinoin also appeared useful in an adolescent female who failed to respond adequately to topical corticosteroids and methotrexate [30] and in two patients who did not tolerate acitretin [104].

Methotrexate — Methotrexate may be given subcutaneously or orally and appears to be effective for GPP:

●Administration and precautions – A typical dose of methotrexate for adults with GPP is 15 mg per week, with a maximum dose of 25 mg per week. Methotrexate is not given daily. We often use subcutaneous, rather than oral, methotrexate given that absorption of oral methotrexate may be reduced at higher doses and bioavailability may be superior with subcutaneous dosing [105-107].

Significant clinical improvement is expected within 8 to 12 weeks.

Myelosuppression is a potential serious adverse effect of methotrexate that warrants careful administration of treatment and laboratory follow-up. The amount of methotrexate used for the initial dose varies among experts, with some experts initiating with a small test dose (eg, 5 mg per week) followed by upward titration of the dose and others initiating at higher doses (eg, 15 mg per week) [108]. When treating older adult patients or patients with renal insufficiency, the initial dose should not exceed 10 mg per week.

Methotrexate is a teratogen and is contraindicated in pregnancy. Additional adverse effects of methotrexate include gastrointestinal distress, hepatotoxicity, and pulmonary toxicity. Methotrexate should be combined with folic acid supplementation (eg, 1 mg of folic acid per day) to decrease hematologic and gastrointestinal toxicity [109]. (See "Major adverse effects of low-dose methotrexate".)

Monitoring protocols for methotrexate treatment vary; in general, a complete blood count with differential and platelets should be performed at baseline and soon after the initiation of methotrexate and following dose increases. While some clinicians perform hematologic testing approximately one week after dose initiation or changes, others (including the author) perform tests after two to four weeks provided the patient is not an older individual and does not have renal insufficiency. Hematologic testing is repeated every two to four weeks during the first few months of treatment and is subsequently tapered to every one to three months. Periodic laboratory monitoring of kidney and liver function is also indicated during methotrexate therapy.

●Efficacy – Data on the efficacy of methotrexate are limited. In a multicenter study in Japan in which community hospitals were sent questionnaires about patients with GPP, efficacy of methotrexate was documented for 76 percent of 41 patients given this therapy alone or in conjunction with other therapies [80]. In addition, in a retrospective study of 63 patients hospitalized for GPP at Mayo Clinic-affiliated hospitals between 1961 and 1989, good responses to methotrexate were reported for three of eight patients with acute GPP and both of the two patients with annular pustular psoriasis [99].

Adjunctive topical therapy — Because of the widespread nature of GPP, topical medications are primarily reserved for adjunctive therapy.

Topical therapies that have seemed useful for adjunctive therapy in case reports are similar to those used in chronic plaque psoriasis and include topical corticosteroids [110], combination therapy with a topical corticosteroid and topical vitamin D analog [111], and topical tacrolimus [112].

Topical corticosteroids are the topical agents we use most frequently. In our experience, use of a medium potency (eg, group 4 (table 1)) topical corticosteroid under occlusion (eg, triamcinolone 0.1% under wet wraps) seems to improve patient comfort and augment the efficacy of topical corticosteroid therapy. (See "Topical corticosteroids: Use and adverse effects", section on 'Potency' and "Evaluation and management of severe refractory atopic dermatitis (eczema) in adults", section on 'Soak and smear/wet wraps'.)

The development of GPP in association with use of topical corticosteroids [113-115] and topical vitamin D analogs [113,116,117] has been reported. We aim to minimize this risk through focusing topical therapy on small areas of persistent or recalcitrant skin involvement [29,77].

Other therapies — GPP may respond to other therapies.

●Other TNF inhibitors – Successful control of GPP during treatment with anti-TNF biologic agents other than infliximab (eg, adalimumab, certolizumab, etanercept) has been reported in small numbers of patients [41,86,118-123].

•In an open-label study in which 10 patients with GPP received adalimumab (40 or 80 mg every other week after an 80 mg initial dose), 7 patients achieved partial or complete improvement by week 16 [84]. In a retrospective study, two of three GPP flares treated with adalimumab progressed to clinical remissions, and clearance of pustules occurred within 7 and 28 days [86]. Of note, there are reports of TNF-alpha inhibitors inducing pustular psoriasis, including GPP [84].

•In an open-label trial in which 22 adults with GPP or erythrodermic psoriasis were randomly assigned to one of two regimens of certolizumab pegol (either 400 mg every two weeks or 400 mg at weeks 0, 2, and 4, followed by 200 mg every two weeks), all seven patients with GPP were assessed as improved or in remission at week 16 [124]. At weeks 2 and 52, six of seven patients achieved the same endpoint.

●Combination therapy – Combination therapy has seemed beneficial for recalcitrant GPP in some patients. Examples include etanercept and cyclosporine [79], infliximab and methotrexate [38,94], adalimumab and acitretin [125], secukinumab and acitretin [101,102], adalimumab and methotrexate [126], infliximab and acitretin [97], secukinumab and colchicine [127], guselkumab and colchicine [127], acitretin and glycyrrhizin (a hepatoprotective constituent of Chinese herbal medicine) [128], and cyclosporine and psoralens plus ultraviolet A (PUVA) photochemotherapy [78]. In addition, children have responded to combination therapy with narrowband ultraviolet B (UVB) and systemic therapies [20,45] as well as infliximab combined with methotrexate [38].

●Systemic glucocorticoids – Although systemic glucocorticoid therapy can lead to rapid improvement in GPP [81], the treatment must be used with caution because systemic glucocorticoids are implicated as potential inciting factors for GPP [77]. In addition, there is concern for the serious side effects from long-term glucocorticoid therapy. Thus, we do not typically use systemic glucocorticoids. In the event that systemic glucocorticoid treatment is given to obtain initial control of GPP, we suggest also initiating a second therapy in an attempt to reduce the likelihood of a disease flare during tapering and discontinuation of the systemic glucocorticoid. However, evidence to support this approach is lacking. The adverse effects of systemic glucocorticoids are reviewed separately. (See "Major adverse effects of systemic glucocorticoids".)

●Other – Case reports document the successful use of granulocyte and monocyte apheresis for the treatment of refractory GPP, including a report of use in a patient harboring the IL36RN mutation [129-136].

Other treatments that have been reported to be effective for GPP in case reports include anakinra [132,137,138], canakinumab [139], investigational gevokizumab (picture 3) [140], mycophenolate mofetil [141], oral zinc [142], dapsone [143], apremilast [144], and topical tacrolimus [145]. Anakinra also appeared beneficial in a small, open-label dose-escalation trial in patients with various forms of pustular psoriasis [146]. It remains to be seen whether anakinra is primarily effective for GPP associated with deficiency of the interleukin (IL) 36 receptor antagonist (DITRA).

GENERALIZED ANNULAR PUSTULAR PSORIASIS — 

Efficacy data for treatments for generalized annular pustular psoriasis are limited. (See 'Disease overview' above.)

Topical corticosteroid therapy may be effective and is often used for initial treatment in children [5]. In addition, topical compresses, wet wraps, or oatmeal baths may be helpful for soothing the skin lesions [5,147].

Topical therapy is less practical when the disease affects a large proportion of the body surface. Disease that cannot be managed with topical therapy alone can be treated with systemic agents. Case reports suggest treatments such as oral retinoids, oral dapsone, methotrexate, and secukinumab may be effective [5,148].

ACRODERMATITIS CONTINUA OF HALLOPEAU — 

Acrodermatitis continua of Hallopeau (ACH) is a rare, chronic, localized form of pustular psoriasis that primarily involves one or more extremity digits (picture 2A and picture 2B-C). ACH is often poorly responsive to therapy. (See "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis", section on 'Acrodermatitis continua of Hallopeau'.)

Treatment options — A variety of local and systemic therapies have been utilized for ACH with variable results. Data on the treatment of this rare disease are primarily limited to case reports and case series.

Topical corticosteroids, topical tacrolimus, topical calcipotriol (alone or in combination with topical corticosteroids or topical tacrolimus), topical mechlorethamine hydrochloride, topical fluorouracil, psoralens plus ultraviolet A (PUVA) photochemotherapy, narrowband ultraviolet B (UVB) phototherapy, ultraviolet A1 (UVA1) phototherapy, and brachytherapy are among the local treatments reported as effective in individual patients with ACH [149-152]. Systemic therapies have included tumor necrosis factor (TNF)-alpha inhibitors, IL-17 inhibitors, IL-23 inhibitors, spesolimab, oral retinoids, methotrexate, cyclosporine, systemic glucocorticoids, methotrexate and propylthiouracil, tocilizumab, and anakinra [149,153-174]. These interventions have not been directly compared in clinical trials, and the best approach to treatment is unclear [165,175].

Our approach — Our initial treatment is typically a superpotent topical corticosteroid (eg, halobetasol or clobetasol), which we instruct the patient to apply to the affected areas once to twice daily for two to four weeks (table 1). Preferably, the patient should apply the medication under an occlusive dressing at night, particularly during the first week of therapy. Plastic wrap is commonly used as an occlusive dressing. Alternatively, occlusion can be applied using a damp cloth covered by dry cloth.

If a good response to treatment occurs, the frequency of application can be tapered as tolerated to a frequency as low as once or twice per week. Alternatively, we prescribe a topical vitamin D analog (eg, topical calcitriol or calcipotriene) in conjunction with the superpotent topical corticosteroid and instruct the patient to apply the topical corticosteroid followed immediately by the vitamin D analog once to twice daily for two to four weeks. Once sufficient improvement is achieved, we taper the topical corticosteroid as tolerated and continue treatment with the vitamin D analog. A commercial product containing both betamethasone dipropionate and calcipotriene is available.

When patients fail to respond adequately to topical therapy, we proceed to targeted phototherapy or systemic therapy and continue to use topical corticosteroids as adjunctive therapy. PUVA photochemotherapy, narrowband UVB, and UVA1 therapy have appeared effective for ACH [149,151,152]. Our preferred systemic therapies include biologic agents (IL-17, IL-23, or TNF inhibitors), spesolimab, or acitretin (0.5 to 1 mg/kg per day), with methotrexate and cyclosporine as alternatives. For patients with severe disease and who are at risk for nail loss, we tend to prescribe biologic therapy.

SOCIETY GUIDELINE LINKS — 

Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Psoriasis".)

SUMMARY AND RECOMMENDATIONS

●Disease overview – Pustular psoriasis may be generalized or localized. (See 'Disease overview' above and "Pustular psoriasis: Pathogenesis, clinical manifestations, and diagnosis".)

•Generalized pustular psoriasis – Generalized pustular psoriasis (GPP) is an uncommon form of psoriasis that typically presents with widespread pustules and erythematous plaques on the skin. (See 'Generalized pustular psoriasis' above.)

-Acute GPP – Acute GPP is a potentially life-threatening disorder with a sudden onset pustular eruption accompanied by fever and malaise (picture 1A-D).

-Generalized annular pustular psoriasis – Generalized annular pustular psoriasis is a less acute disorder with widespread, annular or figurate, erythematous plaques with pustules. Fever may or may not be present.

•Localized pustular psoriasis – Acrodermatitis continua of Hallopeau is a rare, localized form of pustular psoriasis that presents with erythema and localized pustules on the fingers or toes (picture 2A-C). It is controversial whether palmoplantar pustulosis represents an additional variant. (See 'Acrodermatitis continua of Hallopeau' above and "Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis".)

●Management of acute GPP in adults – The management of acute GPP aims to improve skin manifestations, alleviate systemic symptoms, and prevent serious or life-threatening complications. Most patients require systemic therapy (algorithm 1). (See 'Approach to therapy' above.)

•Initial interventions – Initial management should include:

-Assessment of the need for hospitalization

-Review of the patient's medical history for contributory drugs

-Initiation of pharmacologic treatment

-Management of complications (See 'Important interventions' above.)

•Treatment selection – For the initial treatment of acute GPP flares in adults, we suggest intravenous spesolimab rather than other therapies (algorithm 1) (Grade 2C). Our selection of spesolimab is based on randomized trial data that support the efficacy of this drug. Reasonable alternatives include ixekizumab, secukinumab, risankizumab, guselkumab, infliximab, and cyclosporine. Spesolimab has not been proven more effective than these therapies. (See 'Treatment selection' above.)

•Treatment duration – GPP usually has an unstable and prolonged course without treatment. Patients often require long-term systemic therapy. (See 'Treatment duration' above.)

•Treatment for children – Data on the treatment of acute GPP in children are limited. Treatment selection is based on clinical experience and evidence for drug safety. We treat most children with secukinumab or ixekizumab, but other approaches are reasonable. (See 'Children' above.)

●Generalized annular pustular psoriasis – Compared with acute GPP, data on treatments for generalized annular pustular psoriasis are more limited. Topical corticosteroid therapy may be sufficient for patients with relatively limited skin involvement. Patients with extensive skin involvement may benefit from systemic therapy. (See 'Generalized annular pustular psoriasis' above.)

●Acrodermatitis continua of Hallopeau – Acrodermatitis continua of Hallopeau (ACH) is a rare form of localized pustular psoriasis that is often refractory to treatment. For the initial treatment of ACH, we suggest a superpotent topical corticosteroid rather than other treatments (Grade 2C). Patients in whom topical therapy is insufficiently effective may benefit from systemic therapy or phototherapy. (See 'Acrodermatitis continua of Hallopeau' above.)

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Topic 93554 Version 25.0

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67 : Generalized pustular psoriasis flare in a patient affected by plaque psoriasis after BNT162b2 mRNA COVID-19 vaccine, successfully treated with risankizumab.

68 : Generalized Pustular Psoriasis Treated With Risankizumab.

69 : Efficacy and safety of risankizumab in Japanese patients with generalized pustular psoriasis or erythrodermic psoriasis: Primary analysis and 180-week follow-up results from the phase 3, multicenter IMMspire study.

70 : Ustekinumab: effective in a patient with severe recalcitrant generalized pustular psoriasis.

71 : Clearance of annular pustular psoriasis with ustekinumab.

72 : Generalized pustular psoriasis treated with ustekinumab.

73 : Flare of pustular psoriasis after initiating ustekinumab therapy.

74 : Pustular psoriasis development after initial ustekinumab administration in chronic plaque psoriasis.

75 : Paradoxical pustular psoriasis induced by ustekinumab in a patient with Crohn's disease-associated spondyloarthropathy.

76 : Paradoxical flare of pustular psoriasis triggered by ustekinumab, which responded to adalimumab therapy.

77 : Treatment of pustular psoriasis: from the Medical Board of the National Psoriasis Foundation.

78 : Generalized pustular psoriasis responsive to PUVA and oral cyclosporin therapy.

79 : Generalized pustular psoriasis of Zambusch: case report of successful disease control with cyclosporine and etanercept.

80 : Treatments of generalized pustular psoriasis: a multicenter study in Japan.

81 : Therapeutic guidelines for the treatment of generalized pustular psoriasis (GPP) based on a proposed classification of disease severity.

82 : Development of herpes zoster during infliximab treatment for pediatric generalized pustular psoriasis: A case report.

83 : Safety profiles and efficacy of infliximab therapy in Japanese patients with plaque psoriasis with or without psoriatic arthritis, pustular psoriasis or psoriatic erythroderma: Results from the prospective post-marketing surveillance.

84 : Psoriasis and palmoplantar pustulosis associated with tumor necrosis factor-αinhibitors: the Mayo Clinic experience, 1998 to 2010.

85 : Generalized Pustular Psoriasis Induced by Infliximab in a Patient With Inflammatory Bowel Disease.

86 : Efficacy and safety of tumor necrosis factor inhibitors in acute generalized pustular psoriasis.

87 : Acute treatment of generalized pustular psoriasis of von Zumbusch with single-dose infliximab.

88 : Infliximab in recalcitrant generalized pustular arthropatic psoriasis.

89 : Life-threatening pustular and erythrodermic psoriasis responding to infliximab.

90 : Successful treatment of Von Zumbusch pustular psoriasis with infliximab.

91 : Successful treatment of recalcitrant palmoplantar pustular psoriasis with sequential use of infliximab and adalimumab.

92 : Treatment of recalcitrant pustular psoriasis with infliximab: effective reduction of chemokine expression.

93 : Recalcitrant, generalized pustular psoriasis: rapid and lasting therapeutic response to antitumour necrosis factor-alpha antibody (infliximab).

94 : Addition of low-dose methotrexate to infliximab in the treatment of a patient with severe, recalcitrant pustular psoriasis.

95 : Two cases of generalized pustular psoriasis: successful treatment with infliximab.

96 : Biological therapy for pustular psoriasis: a systematic review.

97 : Rapid downregulation of innate immune cells, interleukin-12 and interleukin-23 in generalized pustular psoriasis with infliximab in combination with acitretin.

98 : Japanese guidelines for the management and treatment of generalized pustular psoriasis: The new pathogenesis and treatment of GPP.

99 : Generalized pustular psoriasis. A review of 63 cases.

100 : Clinical analysis of generalized pustular psoriasis in Chinese patients: A retrospective study of 110 patients.

101 : Secukinumab and acitretin as a combination therapy for three clinical forms of severe psoriasis in multi-drug refractory patients: A case series of high efficacy and safety profile.

102 : Combination of secukinumab and acitretin for generalized pustular psoriasis: A case report and review of literature.

103 : Isotretinoin vs etretinate therapy in generalized pustular and chronic psoriasis.

104 : Successful treatment of palmoplantar pustulosis with isotretinoin.

105 : Bioavailability of higher dose methotrexate comparing oral and subcutaneous administration in patients with rheumatoid arthritis.

106 : Methotrexate in rheumatoid arthritis: optimizing therapy among different formulations. Current and emerging paradigms.

107 : Update on subcutaneous methotrexate for inflammatory arthritis and psoriasis.

108 : Methotrexate and psoriasis: 2009 National Psoriasis Foundation Consensus Conference.

109 : The effect of folate supplementation on methotrexate efficacy and toxicity in psoriasis patients and folic acid use by dermatologists in the USA.

110 : Generalized pustular psoriasis of pregnancy treated with narrowband UVB and topical steroids.

111 : Generalized pustular psoriasis and hepatic dysfunction associated with oral terbinafine therapy.

112 : Generalized pustular psoriasis successfully treated with topical tacrolimus.

113 : Generalized pustular psoriasis (von Zumbusch) following the use of calcipotriol and betamethasone dipropionate ointment: a report of two cases.

114 : Subacute annular generalized pustular psoriasis treated with etanercept and cyclosporine combination.

115 : Generalized pustular psoriasis (von Zumbusch) following iatrogenic hypocortisolism.

116 : Generalized pustular psoriasis precipitated by topical calcipotriol cream.

117 : Generalized pustular psoriasis precipitated by topical calcipotriol ointment.

118 : Long-term efficacy of adalimumab in generalized pustular psoriasis.

119 : Successful treatment with adalimumab in a patient with psoriatic arthritis and generalized pustular psoriasis.

120 : Etanercept in the treatment of generalized annular pustular psoriasis.

121 : Etanercept at different dosages in the treatment of generalized pustular psoriasis: a case series.

122 : A case of acute generalized pustular psoriasis of von Zumbusch treated with adalimumab.

123 : [Successful treatment of generalized pustular psoriasis with certolizumab].

124 : Efficacy and Safety of Certolizumab Pegol in Japanese Patients with Generalized Pustular Psoriasis and Erythrodermic Psoriasis: 52-Week Results.

125 : Refractory generalized pustular psoriasis responsive to a combination of adalimumab and acitretin.

126 : Efficacy of adalimumab and methotrexate combination therapy on generalized pustular psoriasis patients unresponsive to infliximab monotherapy due to anti-infliximab antibody development.

127 : Combination therapy with biologic and colchicine for generalized pustular psoriasis.

128 : Combination therapy with acitretin and glycyrrhizin in generalized pustular psoriasis with liver test abnormalities: A case series.

129 : Successful treatment of three cases of generalized pustular psoriasis with granulocyte and monocyte adsorption apheresis.

130 : Combination therapy of infliximab and granulocyte/monocyte adsorption apheresis for refractory pustular psoriasis with psoriatic arthritis.

131 : Immunosuppressant and infliximab-resistant generalized pustular psoriasis successfully treated with granulocyte and monocyte adsorption apheresis.

132 : Therapeutic depletion of myeloid lineage leukocytes in patients with generalized pustular psoriasis indicates a major role for neutrophils in the immunopathogenesis of psoriasis.

133 : Generalized pustular psoriasis successfully treated with granulocyte and monocyte adsorption apheresis.

134 : Granulocyte and Monocyte Adsorption Apheresis for Generalized Pustular Psoriasis: Therapeutic Outcomes in Three Refractory Patients.

135 : Generalized pustular psoriasis caused by deficiency of interleukin-36 receptor antagonist successfully treated with granulocyte and monocyte adsorption apheresis.

136 : A Case of Old Age-Onset Generalized Pustular Psoriasis with a Deficiency of IL-36RN (DITRA) Treated by Granulocyte and Monocyte Apheresis.

137 : Successful treatment of generalized pustular psoriasis with the interleukin-1-receptor antagonist Anakinra: lack of correlation with IL1RN mutations.

138 : Successful therapy with anakinra in a patient with generalized pustular psoriasis carrying IL36RN mutations.

139 : Successful response in a case of severe pustular psoriasis after interleukin-1βinhibition.

140 : Treatment of two patients with generalized pustular psoriasis with the interleukin-1βinhibitor gevokizumab.

141 : Severe generalized pustular psoriasis treated with mycophenolate mofetil.

142 : Dramatic response to oral zinc in a case of subacute form of generalized pustular psoriasis.

143 : Dapsone Therapy for Pustular Psoriasis: Case Series and Review of the Literature.

144 : Generalized pustular psoriasis treated with apremilast in a patient with multiple medical comorbidities.

145 : A case of generalized pustular psoriasis treated with topical tacrolimus.

146 : Anakinra for refractory pustular psoriasis: A phase II, open-label, dose-escalation trial.

147 : Picture of the month--quiz case. Pustular psoriasis, annular type.

148 : Recalcitrant annular pustular psoriasis associated with psoriatic arthritis successfully treated with secukinumab.

149 : Acrodermatitis continua of Hallopeau: evolution of treatment options.

150 : Novel Application of High-Dose-Rate Brachytherapy for Severe, Recalcitrant Acrodermatitis Continua of Hallopeau.

151 : Treatment for palmoplantar pustular psoriasis: systematic literature review, evidence-based recommendations and expert opinion.

152 : UVA1 vs. narrowband UVB phototherapy in the treatment of palmoplantar pustulosis: a pilot randomized controlled study.

153 : Acrodermatitis continua of Hallopeau (ACH): two cases successfully treated with adalimumab.

154 : Efficacy of adalimumab for the treatment of refractory paediatric acrodermatitis continua of hallopeau.

155 : Acitretin- and tumor necrosis factor inhibitor-resistant acrodermatitis continua of hallopeau responsive to the interleukin 1 receptor antagonist anakinra.

156 : A recalcitrant acrodermatitis continua of Hallopeau successfully treated with etanercept.

157 : Efficacy of ustekinumab in palmoplantar pustulosis and palmoplantar pustular psoriasis.

158 : A case of tumour necrosis factor-αinhibitor- and rituximab-induced plantar pustular psoriasis that completely resolved with tocilizumab.

159 : Secukinumab for Acrodermatitis Continua of Hallopeau.

160 : Generalized acrodermatitis continua of Hallopeau with an IL36RN variant successfully treated with bimekizumab.

161 : Plaque psoriasis, generalized pustular psoriasis, palmoplantar pustulosis, and acrodermatitis continua of Hallopeau successfully treated with bimekizumab: a promising therapeutic approach.

162 : Acrodermatitis continua Hallopeau successfully treated by risankizumab.

163 : Effective management of acrodermatitis continua of Hallopeau with guselkumab in a Wiskott-Aldrich syndrome patient.

164 : A case of acrodermatitis continua of Hallopeau successfully treated with guselkumab.

165 : Acrodermatitis continua of hallopeau: aggravating factors and treatment outcomes of 96 patients.

166 : Spesolimab Response in a Girl With Acrodermatitis Continua of Hallopeau.

167 : Spesolimab in refractory paediatric acrodermatitis continua of Hallopeau, a case series.

168 : Rapid improvement in refractory acrodermatitis continua of Hallopeau with spesolimab injection.

169 : Acrodermatitis of Hallopeau and erosive oral mucositis successfully treated with secukinumab.

170 : Ustekinumab for the treatment of acrodermatitis continua of Hallopeau refractory to anti-TNF agents.

171 : Treatment of acrodermatitis continua of hallopeau with ixekizumab.

172 : Treatment of Recalcitrant Acrodermatitis Continua of Hallopeau With Brodalumab

173 : Efficacy and safety of TNF blockers and of ustekinumab in palmoplantar pustulosis and in acrodermatitis continua of Hallopeau.

174 : Acrodermatitis continua of Hallopeau successfully treated with spesolimab.

175 : Treatment of acrodermatitis continua of Hallopeau: A case series of 39 patients.

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